GLYCOGEN STORAGE DISEASE

该研究旨在研究葡萄糖与葡萄糖之间的相互作用， 糖原累积病 该方法包括评价 蒸煮和未蒸煮淀粉的水解和吸收的测定 肝葡萄糖产生和葡萄糖通量的测定 代谢物。 Ⅰ型糖原累积症患儿的反应性调查 疾病的管理不同的生淀粉表明， 水解速率的家族间差异，表明多态性 胰淀粉酶 肝硬化患者肝脏葡萄糖生成率的研究 使用13 C-葡萄糖的I型GSD已经开始。 除了 确定葡萄糖生产率的肝脏，我们将遵循 模式13 C转化为克雷布循环中间体。

The study seeks to investigate the various glucose interactions in the glycogen storage diseases. The approach includes the evaluation of hydrolysis and absorption of cooked and uncooked starches, determination of hepatic glucose production and determination of the flux of glucose metabolites. The investigation of the responses of children with Type I glycogen storage disease to the administration of different raw starches suggests interfamily differences in the rate of hydrolysis, suggesting polymorphism of pancreatic amylase. The study of the rate of glucose production by the liver of patients with Type I GSD using 13C-glucose has been initiated. In addition to determining the rate of glucose production by the liver, we will follow the pattern 13C into the Kreb cycle intermediates.